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Clinical and Laboratory Findings in Patients with δ-Storage Pool Disease: A Case Series

dc.rights.licenseopenen_US
dc.contributor.authorSELLE, Fabien
hal.structure.identifierBiologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases
dc.contributor.authorJAMES, Chloé
hal.structure.identifierBiologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases
dc.contributor.authorTUFFIGO, Marie
hal.structure.identifierBiologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases
dc.contributor.authorPILLOIS, Xavier
hal.structure.identifierBiologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases
dc.contributor.authorVIALLARD, Jean-François
dc.contributor.authorALESSI, Marie-Christine
dc.contributor.authorFIORE, Mathieu
dc.date.accessioned2020-10-28T09:13:41Z
dc.date.available2020-10-28T09:13:41Z
dc.date.issued2016-06
dc.identifier.issn0094-6176, 1098-9064en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/11531
dc.description.abstractEnPlatelet δ-storage pool disease (δ-SPD) is a platelet function disorder characterized by a reduction in the number or content of dense granules. Reports on δ-SPD are mostly limited to case presentations. We aimed to retrospectively describe a series of patients with δ-SPD to better characterize the disease. We studied 16 patients with congenital or acquired δ-SPD. Lumiaggregometry, α- and δ-granules content, platelet ultrastructure, αIIbβ3 integrin, and glycoprotein Ib (GPIb) activation were assessed. Most of the patients generally demonstrate mild to moderate bleeding diathesis. Platelet aggregation studies showed moderate abnormalities with variable profiles, while all the individuals had almost complete absence of adenosine triphosphate release. Mepacrine capture, CD63 expression, and study of dense granules by electron microscopy enabled to distinguish different subtypes of δ-SPD with quantitative or qualitative defect. Surprisingly, significantly decreased GPIb expression levels after platelet activation with thrombin receptor activating peptide 50 μM were found, suggesting that GPIb-impaired mobilization may represent an additional feature of the disorder. In conclusion, δ-SPD represents a complex disorder with various clinical and biological aspects, requiring a great deal of expertise to be properly diagnosed.
dc.language.isoENen_US
dc.subject*Article CLINIQUE
dc.title.enClinical and Laboratory Findings in Patients with δ-Storage Pool Disease: A Case Series
dc.title.alternativeSemin Thromb Hemost.en_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1055/s-0036-1584568en_US
dc.subject.halSciences du Vivant [q-bio]/Médecine humaine et pathologieen_US
bordeaux.journalSeminars in Thrombosis and Hemostasisen_US
bordeaux.page048–058en_US
bordeaux.volume43en_US
bordeaux.hal.laboratoriesBiologie des maladies cardiovasculaires - U1034en_US
bordeaux.issue1en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
hal.exportfalse
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